Kidney transplant in multiple myeloma. Early recurrence in the graft with sustained normal renal function.
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Biomedical subjects
Publications and source records attributed to A Meyrier.
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Prognosis of familial paroxysmal polyserositis is primarily related to the presence of renal insufficiency due to the amyloidosis. Current treatment of terminal renal failure--haemodialysis and renal transplantation--has increased survival in these patients, but prognosis remains relatively poor because of the extrarenal, mainly cardiac deposits which have had time to develop. Preventive treatment is therefore all that can be hoped for, and initial results of the use of colchicine in this amyloid affection, by Israelian authors, appear encouraging.
The authors report a quite exceptional case of isolated angiomyolipoma of one kidney in the absence of any manifestation of phakomatosis. Discovery of the tumour led to special investigations which confirmed the solid and hypervascularised nature of the tumour. A doubt remained with regard to its precise nature, and hence an extended nephrectomy, though without damage to the vessels at the time of section. The kidney was cooled and preserved by perfusion which allowed time for adequate histological study. The diagnosis of angiomyolipoma being confirmed, without the slightest risk of dissemination if by chance it had not been a benign tumour, the kidney was transplanted into the lumbar fossa via its own vessels with an excellent result. The authors discuss two problems on the basis of this case: 1) That of preoperative diagnostic difficulties: the scanner provides valuable information when it reveals the presence of intra-tumoral fatty tissue. 2) Difficulties in management when the benign nature of the lesion cannot be confirmed before histological examination. The solution adopted: routine extended nephrectomy with ex-vivo perfusion of the kidney making it possible to obey the basic rules of oncology. Frozen section biopsy confirms the diagnosis of angiomyolipoma. It is then possible to auto-transplant the kidney after tumorectomy.
We report on 4 cases of reversible acute renal failure (ARF), appearing within the first days after institution of a sulfinpyrazone treatment (600 mg p. day) early after a myocardial infarction. Urinary data were consistent with an acute tubular necrosis. A renal biopsy, performed in 3 patients, revealed only very discrete tubular and interstitial lesions, not helpful in the understanding of the ARF. Three pathogenetic mechanisms could be evoked. Data in favour of an acute tubular precipitation of uric acid or an immunologically induced acute interstitial nephritis are lacking. More probably this ARF is due to an inhibitory effect of sulfinpyrazone on the renal prostaglandin synthesis. Early after a myocardial infarction, renal prostaglandins could play an important protective role in maintaining the renal circulation. Renal function should be monitored closely when sulfinpyrazone is prescribed, especially early after a myocardial infarction.
The glucose tolerance and serum lipids pattern were studied in 20 hemodialyzed patients after 8 and 12 hours of fasting. They all were non-obese uremics under 40 years of age. The test was conducted according to the nutritional conditions of the protocol of de Gennes. Fasting hyperglycemia and an abnormal glucose tolerance test were observed in 75% of the patients. The basal serum insulin levels were increased in 80% of the cases. 40% of the population studied exhibited significant hypertriglyceridemia after 12 hours and 65% after only 8 hours of fasting. Thus, this protocol was useful for detecting abnormalities in the metabolism of triglycerides in hemodialyzed patients with few baseline disturbances. The normal levels of serum triglycerides at 12 hours could be explained by 1) the relatively young age of the population studied and 2) dialysis with glucose free solutions.
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Thirty patients receiving haemodialysis, including two who had undergone bilateral nephrectomy, showed significant increases (p less than 0.001) in packed cell volume, haemoglobin concentration, and reticulocyte count during an episode of viral or toxic liver cytolysis. In 19 cases the episode lasted a mean of 5.0 +/- SD 2.9 months and was accompanied by improved haematological values for a mean of 12.0 +/- 6.0 weeks. Of the remaining patients, in whom the episode lasted a mean of 8.0 +/- 3.5 months (p less than 0.02), seven showed continued improvement a mean of 30.0 +/- 16.0 weeks after cytolysis. In eight patients in this group there was an apparent correlation between persistently increased haemoglobin concentrations and biopsy evidence of chronic hepatitis. The findings suggest that the reduction in anaemia resulted from enhanced red-cell production mediated by secretion of erythropoietin from regenerating liver cells. If confirmed this might have important implications for the treatment of anaemia in chronic renal insufficiency.
A case of multiple myeloma with intra- and extracapillary proliferative glomerulonephropathy and other unusual morphologic and clinical manifestations is described. The probable role of light chain proteins in the pathogenesis of the glomerular lesions is discussed. Evidence is presented documenting the beneficial effects of appropriate therapy on the activity and progression of the renal lesions.
The glucose tolerance and serum lipids pattern were studied in 20 hemodialyzed patients after 8 and 12 hours of fasting. They all were non-obese uremics under 40 years of age. The test was conducted according to the nutritional conditions of the protocol of de Gennes. Fasting hyperglycemia and an abnormal glucose tolerance test were observed in 75% of the patients. The basal serum insulin levels were increased in 80% of the cases. 40% of the population studied exhibited significant hypertriglyceridemia after 12 hours and 65% after only 8 hours of fasting. Thus, this protocol was useful for detecting abnormalities in the metabolism of triglycerides in hemodialized patients with few baseline disturbances. The normal levels of serum tryglycerides at 12 hours could be explained by 1) the relatively young age of the population studied and 2) dialysis with glucose free solutions.
We report on a case of tuberous sclerosis in a 36-year-old female with chronic renal failure, who was treated by hemodialysis and transplantation. The diagnosis of tuberous sclerosis was ascertained by the association of typical cutaneous retinal and osseous lesions. On the contrary, the renal localisations were unusual. Early hypertension was exacerbated in the course of 2 pregnancies but was never severe. It preceded the late onset of renal failure which progressed slowly. The kidneys were small, with a peculiar angiographic aspect: the renal tissue was not invaded by hamartomas but the whole arterial bed was diffusely modified, with a cortical perfusion defect. Both kidneys were studied after bilateral nephrectomy. The lesions were far more complex than a simple replacement of the renal tissue by angiomyolipomas: they involved the vessels, the interstitium (with lipids inclusions surrounded by a macrophagic reaction) and the glomeruli (with focal and segmental sclerosis). This observation (which is the 11th case in the literature of tuberous sclerosis with chronic renal failure and the 3rd treated by transplantation) documents a particular variety of congenital and familial nephropathy with delayed revelation.
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In 23 chronic renal patients serum and urinary concentrations of pipemidic acid were studied, the drug being given orally for 3 to 105 days. In 13 non-dialyzed patients, whose GFR varied from 4.5 to 36 ml/mn the dosage was 11.2 to 30 mg/kg/day. Maximum serum concentrations were comprised between 6 and 29 micrograms/ml. In 2 patients minor clinical side-effects indicated a maximum tolerance level of 25 micrograms/ml. Urinary concentrations remained elevated in spite of severe renal failure. In 10 hemodialyzed patients maximum predialysis levels varied from 5.6 to 28 micrograms/ml. No side-effects were noticed. The dialysance of pipemidic acid was high, due to a low molecular weight and to a binding to proteins which seems to be lower in uremics than in normal subjects. We conclude that pipemidic acid can be utilized in chronic renal patients, in taking advantage of low GFR's for obtaining serum bactericidal concentrations on susceptible organisms.
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Studies of the serological development after 26 renal transplants confirm the high frequency of antibody rises not only against the herpes virus group, but also against other virus groups such as measles, Coxsackie B viruses. These antibody rises correlate with febrile episodes and hepatic dysfunction in which CMV is the most often involved. However, the frequency of antibody rises against various viral antigens without any clinical event to suggest viral etiology; the lack of concomitant virus isolation (except the herpes group), as well as the ocurrence of simultaneous antibody rises against several viruses, all suggest that some of these various antibody rises observed may be related to immunological dysfunction rather than to virus infection.
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